Epidemiology

Not rare, an important cause of new daily persistent headaches among young & middle age individuals

Prevalence: ~1 per 50,000, previously probably underdiagnosed

F:M ~ 2:1, onset in 4th or 5th decadeAssociated with connective tissue

disorders (Marfan, Ehler Danlos)

JAMA 2006.;295(19):2286-96

Etiology & Pathogenesis

Generally caused by spinal CSF leakPrecise cause remains largely unknown,

underlying structural weakness of spinal meninges is suspected

Hx of trivial traumatic events elicited in 1/3Wide variety of dural defects; simple dural

hole, fragile meningeal diverticula, absence of dura cover spinal nerve root

JAMA 2006.;295(19):2286-96

Etiology & Pathogenesis

Decreased CSF volume may be final common pathway in pathophysiology

Altered distribution of craniospinal elasticity due to spinal CSF leak may be final common pathway

So, “spontaneous spinal CSF leak” are preferred terms

JAMA 2006.;295(19):2286-96

Diffuse pachymeningeal (dural) enhancement Bilateral subdural effusion/hematomas Downward displacement of brain Enlargement of pituitary gland Engorgement of dural venous sinuses Prominence of spinal epidural venous plexus Venous sinus thrombosis & isolated cortical vein

thrombosisAJNR 2008.; 29:1164-70

Monroe-Kellie Rule

Sum of volumes of intracranial blood, CSF & cerebral tissue must remain constant in an intact cranium

Loss of CSF can be compensated by increased vascular component or by increased intracranial CSF component

JAMA 2006.;295(19):2286-96

Monroe-Kellie Rule

Accounting for pachymeningeal enhancement, engorged venous structures, pituitary hyperemia and subdural effusions

Subdural hematoma may caused by tearing of bridging veins or rupture of thin wall vessels in subdural zone

Sagging of brain is caused by loss of CSF buoyancy

JAMA 2006.;295(19):2286-96

JAMA 2006.;295(19):2286-96

AJNR 2008.; 29:853-56

Diffuse, uniform thicknessLocated at convexity, along falx cerebri, tentorium & posterior fossa duraDisappears after successful treatment

Diffuse Pachymeningeal, (Dural) Enhancement

Bilateral Subdural Effusion/Hematomas

Incidence: 10-50% Tend to be thin (2-7 mm),

typically occur over supratentorial convexity

Have variable MR signal, depending on protein conc. & presence of blood

Disappear after successful treatment

Downward Displacement of The Brain

Low lying cerebellar tonsils Effacement of prepontine

cistern, flattening of pons against clivus

Effacement of perichiasmatic cistern with bowing of optic chiasm over pituitary fossa

Engorgement of Dural Venous Sinuses

On T1W the middle 1/3 of dominant transverse sinus, shows convex borders

All venous sinuses become engorged The falx & tentorium show marked enhancement

AJNR 2007 ; 28:1489-93

Prominent of Epidural Venous Plexus

AJNR 2009.; 30:147-51

Occasionally patients may even present with a compressive myelopathy due to a prominent

venous epidural plexus.

Spinal Extradural Fluid Collections

From: AJNR 2009.; 30:147-51

Treatment

Many cases resolved spontaneouslyThere is no randomized control trial

evaluation of the treatment optionConservative approach: bed rest, oral

hydration, caffeine intake, use of abdominal binder

JAMA 2006.;295(19):2286-96

Treatment

Mainstay of treatment is epidural blood patch (EBP) - epidural injection of autologous blood into epidural space

Effective in relieving symptoms in ~1/3, presumable by dural temponade and sealing the leak

If unsuccessful, it can be repeated

JAMA 2006.;295(19):2286-96

Treatment

If EBP fail, direct EBP or percutaneous placement of fibrin sealant is recommended Requires knowledge of exact site of CSF leak

Surgical Rx is reserved for Pt who failed nonsurgical Rx

Often successful when focal CSF leak is identified

Ligation or placement of muscle pledget

JAMA 2006.;295(19):2286-96

Pre- post Tx appearance

Left: MRI shows ‘saggin’ brain & large pituitary gland. Right: after Tx & Left: MRI shows ‘saggin’ brain & large pituitary gland. Right: after Tx & symptom resolution the brain & gland have a normal appearance.symptom resolution the brain & gland have a normal appearance.

Pituitary gland changes in Intracranial Hypotension

Pre- & post treatment changes. The pituitary gland was initially enlarged & after Pre- & post treatment changes. The pituitary gland was initially enlarged & after Tx it becomes normal in size.Tx it becomes normal in size.

CT myelographyRadioisotope cisternographyMR myelography MR imagingIntrathecal Gd-enhanced MR

► Most common site of CSF leak reported as the cervicothoracic junction & thoracic area, could be single or multiple sites

Complications

Patient with known intracranial hypotension who rapidly Patient with known intracranial hypotension who rapidly deteriorated shows cerebellar, brainstem & cord infarctions.deteriorated shows cerebellar, brainstem & cord infarctions.

AJNR 2009, doi:10.3174/ajnr.A1749

Intracranial hypotension due to Post op spinal CSF leak

Patient had a tumor resection from the thoracic vertebrae & developed Patient had a tumor resection from the thoracic vertebrae & developed intracranial hypotension found to be due to paraspinal thoracic intracranial hypotension found to be due to paraspinal thoracic

pseudomeningocele.pseudomeningocele.

Intracranial hypotension complicated by cortical vein thrmbosis

Iatrogenic- post LP- intracranial hypotension with cortical Iatrogenic- post LP- intracranial hypotension with cortical vein thrombosis (arrow).vein thrombosis (arrow).

References1. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006;

295(19):2286-962. Diffuse pachymeningeal hyperintensity and subdural effusion/hematoma by FLAIR MRI in

patients with spontaneous intracranial hypotension. AJNR 2008; 29:1164-703. The venous distention sign: a diagnostic sign of intracranial hypotension. AJNR 2008;

28:1489-934. Intradural spinal vein enlargement in intracranial hypotension. AJNR 2005; 26:34-385. Diagnostic criteria for spontaneous spinal CSF leaks and intracranial hypotension. AJNR

2008; 29:853-566. Detection of CSF leak in spinal CSF leak syndrome using MR myelography: correlation with

radioisotope cisternography. AJNR 2008; 29:649-54

7. Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome. AJNR 2008; 29:116-21

8. Diagnostic value of spinal MRI in spontaneous intracranial hypotension syndrome. AJNR 2009; 30:147-51

9. False localizing sign of C1-2 CSF leak in spontaneous intracranial hypotension. J Neurosurg 2004; 100:639-44